Neuroimaging in primary lateral sclerosis

Increased interest in the underlying pathogenesis of primary lateral sclerosis (PLS) and its relationship to amyotrophic lateral sclerosis (ALS) has corresponded to a growing number of CNS imaging studies, especially in the past decade. Both its rarity and uncertainty of definite diagnosis prior to...

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Main Authors: Pioro, EP, Turner, MR, Bede, P
Format: Journal article
Language:English
Published: Taylor & Francis 2021
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author Pioro, EP
Turner, MR
Bede, P
author_facet Pioro, EP
Turner, MR
Bede, P
author_sort Pioro, EP
collection OXFORD
description Increased interest in the underlying pathogenesis of primary lateral sclerosis (PLS) and its relationship to amyotrophic lateral sclerosis (ALS) has corresponded to a growing number of CNS imaging studies, especially in the past decade. Both its rarity and uncertainty of definite diagnosis prior to 4 years from symptom onset have resulted in PLS being less studied than ALS. In this review, we highlight most relevant papers applying magnetic resonance imaging (MRI), magnetic resonance spectroscopy (MRS), and positron emission tomography (PET) to analyzing CNS changes in PLS, often in relation to ALS. In patients with PLS, mostly brain, but also spinal cord has been evaluated since significant neurodegeneration is essentially restricted to upper motor neuron (UMN) structures and related pathways. Abnormalities of cortex and subcortical white matter tracts have been identified by structural and functional MRI and MRS studies, while metabolic and cell-specific changes in PLS brain have been revealed using various PET radiotracers. Future neuroimaging studies will continue to explore the interface between the PLS-ALS continuum, identify more changes unique to PLS, apply novel MRI and MRS sequences showing greater structural and neurochemical detail, as well as expand the repertoire of PET radiotracers that reveal various cellular pathologies. Neuroimaging has the potential to play an important role in the evaluation of novel therapies for patients with PLS.
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spelling oxford-uuid:79ef7bae-3de1-47f8-9684-2355dc7cf4c02022-03-26T20:40:33ZNeuroimaging in primary lateral sclerosisJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:79ef7bae-3de1-47f8-9684-2355dc7cf4c0EnglishSymplectic ElementsTaylor & Francis2021Pioro, EPTurner, MRBede, PIncreased interest in the underlying pathogenesis of primary lateral sclerosis (PLS) and its relationship to amyotrophic lateral sclerosis (ALS) has corresponded to a growing number of CNS imaging studies, especially in the past decade. Both its rarity and uncertainty of definite diagnosis prior to 4 years from symptom onset have resulted in PLS being less studied than ALS. In this review, we highlight most relevant papers applying magnetic resonance imaging (MRI), magnetic resonance spectroscopy (MRS), and positron emission tomography (PET) to analyzing CNS changes in PLS, often in relation to ALS. In patients with PLS, mostly brain, but also spinal cord has been evaluated since significant neurodegeneration is essentially restricted to upper motor neuron (UMN) structures and related pathways. Abnormalities of cortex and subcortical white matter tracts have been identified by structural and functional MRI and MRS studies, while metabolic and cell-specific changes in PLS brain have been revealed using various PET radiotracers. Future neuroimaging studies will continue to explore the interface between the PLS-ALS continuum, identify more changes unique to PLS, apply novel MRI and MRS sequences showing greater structural and neurochemical detail, as well as expand the repertoire of PET radiotracers that reveal various cellular pathologies. Neuroimaging has the potential to play an important role in the evaluation of novel therapies for patients with PLS.
spellingShingle Pioro, EP
Turner, MR
Bede, P
Neuroimaging in primary lateral sclerosis
title Neuroimaging in primary lateral sclerosis
title_full Neuroimaging in primary lateral sclerosis
title_fullStr Neuroimaging in primary lateral sclerosis
title_full_unstemmed Neuroimaging in primary lateral sclerosis
title_short Neuroimaging in primary lateral sclerosis
title_sort neuroimaging in primary lateral sclerosis
work_keys_str_mv AT pioroep neuroimaginginprimarylateralsclerosis
AT turnermr neuroimaginginprimarylateralsclerosis
AT bedep neuroimaginginprimarylateralsclerosis